Cases reported "Contracture"

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1/10. Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: use of allogeneic composite cultured skin grafts.

    Recessive dystrophic epidermolysis bullosa (RDEB) is characterised by progressive childhood hand syndactyly and flexion contractures, which can be managed surgically but require split thickness autografts to facilitate satisfactory postoperative healing. We report on the partial substitution, for autografts, of improved composite cultured skin (CCS) allografts. The structure and preparation of these CCSs is outlined and their application in the course of 16 operations performed on 7 RDEB children with syndactyly and flexor contractures of fingers is described. Hand contractures were released and web spaces were covered with local flaps and split thickness autografts, while adjacent sides of the digits and other areas, as well as donor sites were generally grafted with CCS. Morphologic and functional results with CCS were judged to be good to excellent, the average time to recurrence was increased approximately 2-fold and smaller autografts needed to be used. In addition, healed CCS-treated donor sites could provide superior donor sites for further surgery.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/10. Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix.

    Recessive dystrophic epidermolysis bullosa is an inherited mechanobullous disorder of skin and mucous membranes. The most striking clinical characteristic of the disease is the formation of blisters following trivial trauma. Repeated cycles of blistering and scarring result in gradual encasement of the hand in an epidermal "cocoon." The authors treated an 11-year-old boy with recessive dystrophic epidermolysis bullosa who presented with hand contractures and interdigital pseudosyndactyly. Treatment included release of contractures and application of a biosynthetic dermal analog. This report is a histological analysis of the dermal matrix 1 year after initial placement of the allograft. fibroblasts repopulating the dermal allograft had a normal synthetic phenotype and lacked the myofibroblastic features seen in the ungrafted control biopsy. collagen and elastin in the repopulated dermal allograft had normal dermal orientation and maturity in contrast to the sparse, immature collagen and lack of elastin compared with the dermis of an ungrafted control region. Results of this histological study indicate that treatment of recessive dystrophic epidermolysis bullosa with an acellular human dermal allograft may restore some features of normal dermal architecture. Although the initial results are encouraging, longer follow-up is required before definitive conclusions can be made.
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ranking = 2.80885876409
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/10. Surgical management of hand contractures and pseudosyndactyly in dystrophic epidermolysis bullosa.

    The term epidermolysis bullosa refers to a group of disorders characterized by skin blistering following minor trauma. The hands, because of constant use during normal daily activity, are especially exposed to blistering, with secondary scarring leading to pseudosyndactyly, adduction contracture of the thumb, and flexion or extension contracture of the fingers. The standard surgical approach for the correction of these deformities is based on the incisional release of pseudosyndactyly and contractures, and split skin grafting of the secondary wounds. A simplified approach without skin grafting is presented in this paper. Four children (8 hands) underwent 25 operations over two decades. The results, in terms of postoperative healing and recurrence, were comparable with those of the standard approach involving skin grafting.
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ranking = 2.2250945774695
keywords = epidermolysis bullosa, epidermolysis, bullosa
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4/10. Marjolin's ulcer.

    In 1828 John Nicolas Marjolini characterized ulcer with malignant degeneration which developed in scars after burns, but it occurs under varying clinical conditions. Typical feature is the latent period (on average 30 years). It is encountered in 2 forms: a shallow ulcer or exophytic tumour, most frequently on the lower extremities. At the Prague Burn Centre 11 patients were treated since 1978 till 1998. A unique case was a man suffering from congenital form of epidermolysis bullosa who developed Marjolin's ulcer on his foot (histological examination confirmed well differentiated squamous cell carcinoma). After 4 years he died with extreme cachexia and metastatic spread of the tumour, because he refused repeatedly amputation and lymph nodes dissection. To prevent Marjolin's ulcer several recommendations are presented.
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ranking = 0.44501891549391
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/10. Use of skin substitutes in pediatric patients.

    There are various artificial skin substitutes available commercially. The authors have used Integra, cultured epithelium, and Apligraf in their clinic. In the present report, they present their experiences based on two case reports. The first patient was a 12-year-old boy with widespread skin defects and left axillary contracture due to epidermolysis bullosa (EB). Apligraf was used to cover the skin defects on the trunk and face and to manage ectropion and axillary contracture. The second patient was a 6-year-old boy who suffered neurocutaneous melanosis. Partial excision of a pigmented lesion on the back created a large defect. Integra application followed by repair with cultured autologous skin was accomplished, and the results were satisfactory. skin substitute products 1) are commercially immediately available; 2) are effective for management of contractures, chronic wounds, and chronic skin illnesses; 3) decrease or avoid the risk of donor area morbidity, which is more difficult to treat in children; 4) provide long-term coverage of the wound; and 5) can be used in conjunction with autologous tissue (e.g., Integra followed by cultured epithelium applications).
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ranking = 0.44501891549391
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/10. Anesthetic management of a difficult airway in a patient with epidermolysis bullosa: a case report.

    Epidermolysis bullosa is an inherited skin disease that leads to an array of medical problems. patients are susceptible to blistering and scar formation following even minor trauma. These patients may present with scarring, limiting the range of motion of their temporal mandibular joint. This case report describes a 15-year-old patient with epidermolysis bullosa presenting for contracture release, with a difficult airway.
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ranking = 2.250624305157
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/10. Management of advanced foot deformities in dystrophic epidermolysis bullosa.

    Treatment for the characteristic hand deformities of dystrophic epidermolysis bullosa has been well described in the literature. We present our first experience delineating the surgical treatment for advanced foot deformities in this disease. This case emphasizes the need for release of contractures, the use of split-thickness skin grafts, and the need for rigid fixation to maintain surgical correction until healing is complete.
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ranking = 2.2250945774695
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/10. Epidermolysis bullosa: current concepts and management of the advanced hand deformity.

    A current classification of the types of epidermolysis bullosa is reviewed, in conjunction with some etiological concepts. The pathomechanics of the advanced hand deformity are discussed, and applied to a sequential plan of reconstructive surgery. The objective is the restoration of elementary hand function. Two illustrative cases are presented.
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ranking = 0.54713782624384
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/10. The spectrum of epidermolysis bullosa acquisita.

    A patient has an acquired, scarring, bullous eruption. The severity of the cutaneous, ocular, esophageal, and laryngeal scarring was suggestive of cicatricial pemphigoid or the severely dystrophic forms of epidermolysis bullosa. Clinical features and histologic and immunofluorescence and electron microscopic study led to the diagnosis of epidermolysis bullosa acquisita. The spectrum of epidermolysis bullosa acquisita includes cases that closely approximate the severity of disease previously recognized only for the recessive form of epidermolysis bullosa dystrophica.
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ranking = 3.5601513239513
keywords = epidermolysis bullosa, epidermolysis, bullosa
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10/10. A case of dystrophic epidermolysis bullosa: surgical treatment for hand contracture using abdominal skin flap.

    The patient was a 15-year-old male. Since birth, he has suffered from recurrent blistering, especially on the extremities. histology and electron microscopic finding of the cutaneous lesions were compatible with those of dystrophic epidermolysis bullosa. contracture and mobility limitation of the hands had gradually deteriorated. Prior to the admission to our clinic, both hands' contracture had been surgically treated 4 times in another hospital; however, after treatment with skin grafting, hand contracture recurred each time within one year. This time, surgical treatment was carried out for his left hand contracture using an abdominal skin flap under local anesthesia. In comparison with the free skin graft the abdominal skin flap improved hand contracture for a longer time. Abdominal skin flap is recommended as a method for the treatment of hand contracture of patients with dystrophic epidermolysis bullosa.
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ranking = 2.6701134929634
keywords = epidermolysis bullosa, epidermolysis, bullosa
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